Living with PH Plus... Scimitar Syndrome Rita Simone Hébert's Story

Although my lung pressures were measured throughout my life, I had never heard the term “pulmonary hypertension” until 2004. That is the year my (then) cardiologist announced that absolutely nothing more could be done for me medically. That dire statement led me to do a little online research regarding PH. That is also how I found a fantastic PH specialist here in Montreal, who has been monitoring me closely since early 2005.

Some of my early memories as a young child involve breathlessness. I am no stranger to rare conditions such as PH. I was born with an even rarer disorder called a scimitar syndrome. A scimitar is a short sword with a curved blade that broadens toward the point, used originally in Eastern countries during the 16th century. Patients who have this cardiopulmonary defect have a shadow on a lung, which resembles a scimitar. Not all patients diagnosed with this syndrome develop PH. I did!

Due to this heart-lung malformation, I have never had the luxury of experiencing a normal existence. I have never known what it feels like to be able to run (or jog) more than a few meters or keep up with my peers on a bicycle. As a child I spent a lot of time in the local Children’s Hospital where I had my first four heart catheterizations. The biggest heartbreaker, however, was when I found out that I should not have children, despite the corrective surgery I underwent at the age of 23.

I wish I could say that “all’s well that ends well” at this point. However, the difficult part of living with this malformation is that I am not a candidate for most PH drugs. I’ve had to have two more corrective surgeries in the past two years. The first one was a noninvasive pulmonary dilation, followed by open-heart surgery in December 2011.

Although not “all’s well that ends well” yet, living with PH, as well as with this condition, has led me to come across some exceptional people and circumstances. During this PH journey, I have been treated by the most caring and dedicated PH specialist on the globe, Dr. David Langleben. His availability and generosity of spirit surpasses any expectations I could possibly have imagined!

As a patient at his clinic, I also get to see Lyda Lesenko, our local PH nurse coordinator, who we aptly nicknamed “our guardian angel.” Lyda has gone way beyond the call of duty on numerous occasions. For example, Lyda was instrumental in setting up a bilingual support group for the patients at our clinic. As a result of these support group meetings, I feel privileged to have met some of the most outstandingly courageous patients and caregivers. Not only are they bravely proactive in the face of this disease, they are also major players in helping to create support and awareness locally and nationally.

During this PH journey, I also came across a great self-help program called “How to Live a Healthy Life With a Chronic Illness.” This program, which was initially developed at Stanford University, is now taught worldwide in several different languages. I was so taken by its simplicity and effectiveness that I trained to become a program leader. I’m also proud to add that I now teach it in French and English here in Montreal. Read more information on this program

Other lessons I’ve learned along the PH path include not letting the things I cannot do prevent me from doing the things I can. I also learned that being “my personal best” in all matters, given my physical limitations, is all that really matters. I may not be able to run a mile, but perhaps I can still dance a little … in the rain … to the sound of my own tune! I wish you all to do the same.

Wishing you all my personal best!

By Rita Simone Hébert,
Patient with secondary PH due to a complex heart-lung malformation
This article first appeared in Fall 2012 Pathlight.